临床上表现为快速进展性痴呆的血管内大B细胞淋巴瘤。
Intravascular large B-cell lymphoma presenting clinically as rapidly progressive dementia.
作者信息
Brett F M, Chen D, Loftus T, Langan Y, Looby S, Hutchinson S
机构信息
Department of Clinical Neurological Sciences, RCSI, Dublin 9, Ireland.
Department of Clinical Neurological Sciences, Beaumont Hospital, Dublin, Ireland.
出版信息
Ir J Med Sci. 2018 May;187(2):319-322. doi: 10.1007/s11845-017-1653-5. Epub 2017 Jul 19.
BACKGROUND
In patients presenting with rapidly progressive dementia, prion disease may enter the differential diagnosis. The commonest malignancies masquerading as prion disease are primary CNS lymphoma and intravascular large B-cell lymphoma, both rare and difficult to diagnose without brain biopsy.
CASE PRESENTATION
This 82-year-old lady with a past history of hypertension, presented with rapidly progressive cognitive impairment and ataxia. The possibility of sCJD was raised. Brain biopsy was carried out. Western blot for prion protein was negative. Brain biopsy showed intravascular large B-cell lymphoma. She died shortly afterwards.
CONCLUSION
The clinical presentation of intravascular large B-cell lymphoma is diverse. Patients may present as in this case with dementia, seizures, and myoclonus leading to a clinical diagnosis of sCJD. The diagnosis here was made at biopsy but is made at autopsy in over 50% of cases.
背景
在出现快速进展性痴呆的患者中，朊病毒病可能会进入鉴别诊断范围。伪装成朊病毒病的最常见恶性肿瘤是原发性中枢神经系统淋巴瘤和血管内大B细胞淋巴瘤，这两种肿瘤都很罕见，且在没有脑活检的情况下很难诊断。
病例介绍
这位82岁的女性有高血压病史，出现了快速进展的认知障碍和共济失调。散发性克雅氏病的可能性被提出。进行了脑活检。朊病毒蛋白的western印迹检测为阴性。脑活检显示为血管内大B细胞淋巴瘤。她随后不久死亡。
结论
血管内大B细胞淋巴瘤的临床表现多样。患者可能如本病例一样表现为痴呆、癫痫发作和肌阵挛，从而导致散发性克雅氏病的临床诊断。此处的诊断是通过活检做出的，但在超过50%的病例中是在尸检时做出的。
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